期刊
MITOCHONDRION
卷 13, 期 6, 页码 801-809出版社
ELSEVIER SCI LTD
DOI: 10.1016/j.mito.2013.05.006
关键词
Huntington disease; Platelet; Mitochondria; Adenine nucleotides; Mutant huntingtin; Mitochondrial respiratory chain complexes
资金
- Instituto de Investigacao Inter-disciplinar (III), Universidade de Coimbra, Portugal [III/BIO/49/2005]
- Gabinete de Apoio a Investigacao, Faculdade de Medicina, Universidade de Coimbra, Portugal [STARTER S-09]
- Fundacao para a Ciencia e a Tecnologia (FCT), Portugal [PEst-C/SAU/LA0001/2011, PTDC/SAU-FCF/108056/2008, BD/30147/2006, SFRH/BPD/26872/2006]
- POPH - 'Programa Operacional Potencial Humano'
- QREN - 'Quadro de Referencia Estrategico Nacional'
- Fundo Social Europeu da Uniao Europeia
- Fundação para a Ciência e a Tecnologia [SFRH/BPD/26872/2006] Funding Source: FCT
Mitochondrial dysfunction has been implicated in Huntington's disease (HD) pathogenesis. We analyzed the activity of mitochondrial complexes (Cx) I-IV, protein levels of selected Cx subunits and adenine nucleotides in platelet mitochondria from pre-symptomatic versus symptomatic HD human carriers and age-matched control individuals. Mitochondrial platelets exhibited reduced activity of citrate synthase in pre-symptomatic and Cx-I in pre-symptomatic and symptomatic HD carriers. Positive correlation between Cx activity and protein subunits was observed for Cx-I in symptomatic HD patient's mitochondria. Moreover, AMP increased in mitochondria from pre-symptomatic HD carriers. Results highlight mitochondrial changes occurring before the onset of HD clinical symptoms. (C) 2013 Elsevier B.V. and Mitochondria Research Society. All rights reserved.
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